Other

Infections
Fournier Gangrene
Fournier gangrene is a potentially life-threatening form of necrotizing
fasciitis involving the male genitalia. It is also known as idiopathic gangrene of
the scrotum, streptococcal scrotal gangrene, perineal phlegmon, and spontaneous
fulminant gangrene of the scrotum (Fournier, 1883, 1884). As originally reported
by Baurienne in 1764, then by Fournier in 1883, it was characterized by an
abrupt onset of a rapidly fulminating genital gangrene of idiopathic origin in
previously healthy young patients that resulted in gangrenous destruction of the
genitalia. The disease now differs from these descriptions in that it involves a
broader age range, including older patients (Bejanga, 1979; Wolach et al., 1989),
follows a more indolent course, and has a less abrupt onset; and, in
approximately 95% of the cases, a source can now be identified (Burpee and
Edwards, 1972; Jamieson et al., 1984; Kearney and Carling, 1983; Macrea,
1945; Spirnak et al., 1984).
Infection most commonly arises from the skin, urethra, or rectal regions.
An association between urethral obstruction associated with strictures and
extravasation and instrumentation has been well documented. Predisposing
factors include diabetes mellitus, local trauma, paraphimosis, periurethral
extravasation of urine, perirectal or perianal infections, and surgery such as
circumcision or herniorrhaphy. In cases originating in the genitalia,
specifically as a result of urethral obstruction, the infecting bacteria
probably pass through Buck fascia of the penis and spread along the Dartos
fascia of the scrotum and penis, Colles fascia of the perineum, and Scarpa
fascia of the anterior abdominal wall. In view of the typical foul odor
associated with this condition, a major role for anaerobic bacteria is likely.
Wound cultures generally yield multiple organisms, implicating anaerobic-
aerobic synergy (Cohen, 1986; Meleney, 1933; Miller, 1983). Mixed cultures
containing facultative organisms (E. coli, Klebsiella, enterococci) along with
anaerobes (Bacteroides, Fusobacterium, Clostridium, microaerophilic
streptococci) have been obtained from the lesions.

Clinical Presentation
Patients frequently have a history of recent perineal trauma,
instrumentation, urethral stricture associated with sexually transmitted
disease, or urethral cutaneous fistula. Pain, rectal bleeding, and a history of
anal fissures suggest a rectal source of infection. Dermal sources are suggested
by history of acute and chronic infections of the scrotum and spreading recurrent
hidradenitis suppurativa or balanitis.
The infection commonly starts as cellulitis adjacent to the portal of entry.
Early on, the involved area is swollen, erythematous, and tender as the
infection begins to involve the deep fascia. Pain is prominent, and fever and
systemic toxicity are marked (Paty and Smith, 1992). The swelling and
crepitus of the scrotum quickly increase, and dark purple areas develop and
progress to extensive gangrene. If the abdominal wall becomes involved in an
obese patient with diabetes, the process can spread very rapidly. Specific GU
symptoms associated with the condition include dysuria, urethral discharge, and
obstructed voiding. Alterations in mental status, tachypnea, tachycardia, and
temperature greater than 38.3°C (101°F) or less than 35.6°C (96°F) suggest
gram-negative sepsis.

Laboratory Diagnosis and Radiologic Findings

Anemia occurs secondary to a decreased functioning erythrocyte mass caused by
thrombosis and ecchymosis coupled with decreased production secondary to
sepsis (Miller, 1983). Elevated serum creatinine levels, hyponatremia, and
hypocalcemia are common. Hypocalcemia is believed to be secondary to
bacterial lipases that destroy triglycerides and release free fatty acids that chelate
calcium in its ionized form.
Because crepitus (subcutaneous gas) is often an early finding, a plain film
of the abdomen may be helpful in identifying air. Scrotal ultrasonography is
also useful in this regard. Biopsy of the base of an ulcer is characterized by
superficially intact epidermis, dermal necrosis, vascular thrombosis, and
polymorphonuclear leukocyte invasion with subcutaneous tissue necrosis.
Stamenkovic and Lew (1984) noted that the use of frozen sections within 21
hours after the onset of symptoms could confirm a diagnosis earlier and lead to
early institution of appropriate treatment.

Management
Prompt diagnosis is critical because of the rapidity with which the process
can progress. The clinical differentiation of necrotizing fasciitis from cellulitis
may be difficult because the initial signs including pain, edema, and erythema
are not distinctive. However, the presence of marked systemic toxicity out of
proportion to the local finding should alert the clinician. Intravenous
hydration and antimicrobial therapy are indicated in preparation for surgical
debridement. Antimicrobial regimens include broad-spectrum antibiotics (β-
lactam plus β-lactamase inhibitor) such as piperacillin-tazobactam (especially if
Pseudomonas is suspected), ampicillin plus sulbactam, or vancomycin or
carbapenems plus clindamycin or metronidazole (Morpurgo and Galandiuk,
2002).
Immediate debridement is essential. In the patient in whom diagnosis is
clearly suspected on clinical grounds (deep pain with patchy areas of surface
hypoesthesia or crepitation, or bullae and skin necrosis), direct operative
intervention is indicated. Extensive incision should be made through the skin
and subcutaneous tissues, going beyond the areas of involvement until
normal fascia is found. Necrotic fat and fascia should be excised, and the
wound should be left open. A second procedure 24 to 48 hours later is
indicated if there is any question about the adequacy of initial debridement.
Orchiectomy is almost never required because the testes have their own blood
supply independent of the compromised fascial and cutaneous circulation to the
scrotum. Suprapubic diversion should be performed in cases in which
urethral trauma or extravasation is suspected. Colostomy should be
performed if there is colonic or rectal perforation. Hyperbaric oxygen therapy
has shown some promise in shortening hospital stays, increasing wound healing,
and decreasing the gangrenous spread when used in conjunction with
debridement and antimicrobials (Paty and Smith, 1992). Once wound healing is
complete, reconstruction (e.g., using myocutaneous flaps) improves cosmetic
results.

Outcome
The mortality rate averages approximately 20% (Baskin et al., 1990; Clayton
et al., 1990; Cohen, 1986) but ranges from 7% to 75%. Higher mortality rates are
found in patients with diabetes, alcoholics, and those with colorectal sources of
infection who often have a less typical presentation, greater delay in diagnosis,
and more widespread extension. Regardless of the presentation, Fournier
gangrene is a true urologic emergency that demands early recognition,
aggressive treatment with antimicrobial agents, and surgical debridement to
reduce morbidity and mortality.